(Q35686212)

English

Pharmacological Chaperones and Coenzyme Q10 Treatment Improves Mutant β-Glucocerebrosidase Activity and Mitochondrial Function in Neuronopathic Forms of Gaucher Disease

scientific article published on 5 June 2015

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scholarly article

1 reference

Europe PubMed Central

PubMed publication ID

9 August 2017

Pharmacological Chaperones and Coenzyme Q10 Treatment Improves Mutant β-Glucocerebrosidase Activity and Mitochondrial Function in Neuronopathic Forms of Gaucher Disease (English)

1 reference

Europe PubMed Central

PubMed publication ID

9 August 2017

0 references

molecular chaperones

0 references

Gaucher's disease

1 reference

based on heuristic

inferred from title

Mario De la Mata

1

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Juan Garrido-Maraver

4

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Mario D Cordero

5

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Elísabet Alcocer-Gómez

8

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Patricia Ybot-gonzalez

10

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

José Manuel García Fernández

13

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

José A Sánchez-Alcázar

14

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

author name string

David Cotán

2

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Manuel Oropesa-Ávila

3

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Marina Villanueva Paz

6

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Ana Delgado Pavón

7

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Isabel de Lavera

9

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Ana Paula Zaderenko

11

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Carmen Ortiz Mellet

12

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

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publication date

5 June 2015

1 reference

Europe PubMed Central

PubMed publication ID

9 August 2017

Scientific Reports

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Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

5

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

10903

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

copyright license

Creative Commons Attribution 4.0 International

5 June 2015

1 reference

April 2022 Public Data File from Crossref

copyright status

0 references

describes a project that uses

1 reference

Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC4456666/fullTextXML

based on heuristic

inferred from PubMed Central ID database lookup

https://scigraph.springernature.com/pub.10.1038/srep10903

0 references

Clinical applications of coenzyme Q10.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Treatment effect of coenzyme Q(10) and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Mitochondria and quality control defects in a mouse model of Gaucher disease--links to Parkinson's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Bicyclic derivatives of L-idonojirimycin as pharmacological chaperones for neuronopathic forms of Gaucher disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

A multicenter study of glucocerebrosidase mutations in dementia with Lewy bodies

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Pharmacological chaperones as therapeutics for lysosomal storage diseases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Ambroxol as a pharmacological chaperone for mutant glucocerebrosidase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Ceramide targets autophagosomes to mitochondria and induces lethal mitophagy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Autophagy in lysosomal storage disorders

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Impaired parkin-mediated mitochondrial targeting to autophagosomes differentially contributes to tissue pathology in lysosomal storage diseases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Mitochondria and the autophagy-inflammation-cell death axis in organismal aging

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Exploring the link between glucocerebrosidase mutations and parkinsonism

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Pharmacological chaperone therapy for Gaucher disease: a patent review.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Bicyclic (galacto)nojirimycin analogues as glycosidase inhibitors: effect of structural modifications in their pharmacological chaperone potential towards β-glucocerebrosidase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

The proteome of lysosomes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Regulation of autophagy by ROS: physiology and pathology

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Treating lysosomal storage diseases with pharmacological chaperones: from concept to clinics

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Multicenter analysis of glucocerebrosidase mutations in Parkinson's disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Mutations for Gaucher disease confer high susceptibility to Parkinson disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Movement disorders caused by medical disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Coenzyme Q deficiency triggers mitochondria degradation by mitophagy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Randomized, controlled trial of miglustat in Gaucher's disease type 3.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Molecular basis for beta-glucosidase inhibition by ring-modified calystegine analogues

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

The in-depth evaluation of suspected mitochondrial disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Selective degradation of mitochondria by mitophagy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

ER stress and diseases.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Therapeutic strategies to ameliorate lysosomal storage disorders--a focus on Gaucher disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

A breach in the blood-brain barrier

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Protein measurement with the Folin phenol reagent

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Gaucher disease and the clinical experience with substrate reduction therapy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Ubiquinone (coenzyme q10) and mitochondria in oxidative stress of parkinson's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Dissection of autophagosome formation using Apg5-deficient mouse embryonic stem cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Gaucher disease: gene frequencies and genotype/phenotype correlations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Bcl-xL regulates the membrane potential and volume homeostasis of mitochondria

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

Cerebrosides and psychosine disrupt mitochondrial functions.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

12 July 2018

A mutation in the human glucocerebrosidase gene in neuronopathic Gaucher's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

27 July 2018

Targeted delivery of pharmacological chaperones for Gaucher disease to macrophages by a mannosylated cyclodextrin carrier.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

25 September 2018

Cerebrospinal fluid concentrations of idebenone in Friedreich ataxia patients

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

25 September 2018

Synthesis and evaluation of calystegine B2 analogues as glycosidase inhibitors

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4456666

25 September 2018

Potent aminocyclitol glucocerebrosidase inhibitors are subnanomolar pharmacological chaperones for treating gaucher disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Tuning glycosidase inhibition through aglycone interactions: pharmacological chaperones for Fabry disease and GM1 gangliosidosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Biochemical and molecular investigations in respiratory chain deficiencies

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Specific delay in the degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis is derived from cellular proteolytic dysfunction rather than structural alteration of subunit c

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

In vitro characterization of anti-glucosylceramide rabbit antisera

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Design and synthesis of highly potent and selective pharmacological chaperones for the treatment of Gaucher's disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Glucosylceramide accumulation is not confined to the lysosome in fibroblasts from patients with Gaucher disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Secondary coenzyme Q10 deficiency triggers mitochondria degradation by mitophagy in MELAS fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/26045184

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1038/SREP10903

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

Dimensions Publication ID

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PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:26045184%20AND%20SRC:MED&resulttype=core&format=json

12 June 2020

ResearchGate publication ID

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